Rolling mouse Nagoya is an ataxic mutant mouse that carries a mutation in a gene encoding for the alpha 1A subunit of the voltage-gated P/Q-type Ca2+ channel (Cav2.1). This report summarizes our studies and others concerning cerebellar abnormalities in rolling mice based on chemical neuroanatomy. While there are no obvious ...

The myelin sheaths that surround the thick axons of the peripheral nervous system are produced by the highly specialized Schwann cells. Differentiation of Schwann cells and myelination occur in discrete steps. Each of these requires coordinated expression of specific proteins in a precise sequence, yet the regulatory mechanisms controlling protein ...

To describe the phenotypic changes of Schwann cells during nerve regeneration, we made two different neurorrhaphy models after rat sciatic nerves injury, the epineurium neurorrhaphy and small gap bridging suture. Then, at selected time points after surgery (1d, 3d, 5d, 7d, 2 weeks, 3 weeks), the materials were drawn for ...

Solanum bonariense intoxication is characterized by cerebellar neuronal vacuolation, degeneration, and necrosis. Cerebellar Purkinje cells seem especially susceptible, but more research is needed to determine the pathogenesis of neuronal necrosis and the mechanism of Purkinje cell susceptibility. Calbindin D28k (CbD28k) is highly expressed in Purkinje cells and has been used ...

Bradford Barry M BM The Roslin Institute and Royal (Dick) School of Veterinary Studies, University of Edinburgh, Roslin, Midlothian EH25 9PS, United - - 2009

Expression of the prion protein (PrP(C)) is a requirement for host susceptibility to the transmissible spongiform encephalopathies (TSEs) and thought to be necessary for the replication and transport of the infectious agent. The mechanism of TSE neuroinvasion is not fully understood, although the routing of infection has been mapped through ...

Anisotropic tissue structures provide guidance for navigating neurons in vitro and in vivo. Here we optimized the generation of comparable anisotropic monolayers of astrocytes, endothelial cells, and Schwann cells as a first step toward determining which properties of anisotropic cells are sufficient for nerve guidance. The statistical experimental design method ...

Recent evidence showed that neurotransmitters are synthesised in glial cells, such as the Schwann cells, which form myelin sheaths in the PNS. While the presence of GABA type A (GABA-A) receptors has been previously demonstrated in these cells, the evidence of GABA synthesis remained still elusive. In an attempt to ...

Myelinating cocultures of Schwann cells and dorsal root ganglion neurons are a powerful experimental system for probing the molecular mechanisms of axon-Schwann cell interaction. The isolation of a pure population of myelination-competent Schwann cells is a prerequisite for this experimental system. We describe here a protocol for a FACS-based isolation ...

The morphological development of in vitro single cerebellar Purkinje cells obtained from wild type P1 CD1 mice was assessed through a dedicated non-invasive technique based on image processing algorithms and multivariate analysis. Image processing algorithms were implemented to extract metrical features characterizing cell structure and dendritic arborization from sequential optical ...

Recent findings suggested that inducing neural cell adhesion molecule polysialylation in rodents is a promising strategy for promoting tissue repair in the injured central nervous system. Since autologous grafting of Schwann cells is one potential strategy to promote central nervous system remyelination, it is essential to show that such a ...

Johnson et al report tumor formation following murine neural precursor cell transplantation in a rat peripheral nerve injury model, emphasizing the importance of full in vitro characterization of cells prior to transplantation. Cell lines can change during expansion and subclones which may become tumerogenic may be selected in the process ...

Cerebellar unipolar brush cells (UBCs) are glutamatergic interneurons of the granular layer. Previous studies have identified three distinct UBC subsets in the mouse cerebellar cortex: one expressing the calcium-binding protein calretinin (CR), a second expressing both the metabotropic glutamate receptor (mGluR)1alpha and phospholipase C(PLC)beta4, and a third expressing PLCbeta4 but ...

Neural crest (NC) cells are a multipotent, highly migratory cell population that generates most of the components of the peripheral nervous system (PNS), including the glial Schwann cells (SC) and boundary cap (BC) cells. These latter cells are located at the interface between the central nervous system and PNS, at ...

Src-suppressed protein kinase C substrate (SSeCKS) plays an important role in the differentiation process. In regeneration of sciatic nerve injury, expression of SSeCKS decreases, mainly in Schwann cells. However, the function of SSeCKS in Schwann cells differentiation remains unclear. We observed that SSeCKS was decreased in differentiated Schwann cells. In ...

Purkinje cells are specialized for rapid propagation in the heart. Furthermore, Purkinje fibers as the source as well as the perpetuator of arrhythmias is a familiar finding. This is not surprising considering their location in the heart and their unique cell ultrastructure, cell electrophysiology, and mode of excitation-contraction coupling. This ...

Glial cell line-derived neurotrophic factor (GDNF) has a neuroprotective role in Purkinje cells of cerebellum, promoting the survival and the differentiation of these cells. Its signalling is mediated by a receptorial complex GFRalpha1/RET. In the brain of adult zebrafish (Danio rerio) we previously investigated GDNF expression and localization, but no ...

Peripheral nerve lesions are considered the most relevant symptoms of leprosy, a chronic infectious disease caused by Mycobacterium leprae. The strategies employed by M. leprae to infect and multiply inside Schwann cells (SCs), however, remain poorly understood. In this study, it is shown that treatment of SCs with M. leprae ...

Phenotypic remodeling of Schwann cells is required to ensure successful regeneration of damaged peripheral axons. After nerve damage, Schwann cells produce an over 100-fold increase in metalloproteinase-9 (MMP-9), and therapy with an MMP inhibitor increases the number of resident (but not infiltrating) cells in injured nerve. Here, we demonstrate that ...

GOTO cells, a neuroblastoma cell line retaining the ability to differentiate into neuronal or Schwann cells, were found to be rich in membrane rafts containing ganglioside GM2 and hypersensitive to lipid raft-disrupting methyl-beta-cyclodextrin (MbetaCD); the GM2-rich rafts and sensitivity to MbetaCD were markedly diminished upon their differentiation into Schwann cells. ...

Mutations in the frataxin gene cause dorsal root ganglion demyelination and neurodegeneration, which leads to Friedreich's ataxia. However the consequences of frataxin depletion have not been measured in dorsal root ganglia or Schwann cells. We knocked down frataxin in several neural cell lines, including two dorsal root ganglia neural lines, ...

Transmitter release from boutons along a common axon is often regulated depending on the postsynaptic target. Here, GABA release from cerebellar stellate cells onto Purkinje cells and other stellate cells was examined in acute cerebellar slices of 2- and 4-week-old mice. Consistent with previous findings on action potential-dependent GABA release, ...

Neurod1 is a crucial basic helix-loop-helix gene for most cerebellar granule cells and mediates the differentiation of these cells downstream of Atoh1-mediated proliferation of the precursors. In Neurod1 null mice, granule cells die throughout the posterior two thirds of the cerebellar cortex during development. However, Neurod1 is also necessary for ...

INTRODUCTION: Immunohistochemical studies of paraneoplastic cerebellar degeneration (PCD) are rare, and the findings vary. MATERIALS AND METHODS: We performed morphological and immunohistochemical characterization of the brain, medulla and tumour of two patients with PCD, Yo antibodies and ovarian adenocarcinoma. RESULTS: The cerebellum of both patients had extensive loss of Purkinje ...

Woodhoo Ashwin A Department of Cell and Developmental Biology, University College London, - - 2009

Notch signaling is central to vertebrate development, and analysis of Notch has provided important insights into pathogenetic mechanisms in the CNS and many other tissues. However, surprisingly little is known about the role of Notch in the development and pathology of Schwann cells and peripheral nerves. Using transgenic mice and ...

The value of peripheral nerve biopsy is now sometimes questioned due to the high complication rate and the recent development of noninvasive molecular techniques for diagnosis of hereditary neuropathy. However, the disorders that can be diagnosed by genetic analysis are limited and sural nerve biopsy is still a powerful tool ...

Schwann cells (SCs) and olfactory ensheathing glia (OEG) have both been used as cellular transplants to promote spinal cord repair. Both cell types support axonal regeneration and have beneficial effects on functional recovery. A significant difference between SCs and OEG is the effect of these cell types on astrocytes (ACs) ...

CD44 is a multifunctional cell surface glycoprotein which regulates cell-cell and cell-matrix interactions in a variety of tissues. In particular, the protein was found to be expressed in glial cells of developing, but not adult, peripheral nerves, where it takes part in signaling mediated by ErbB class of receptors for ...

The hereditary ataxias are a complex group of neurological disorders characterized by the degeneration of the cerebellum and its associated connections. The molecular mechanisms that trigger the loss of Purkinje cells in this group of diseases remain incompletely understood. Here, we report a previously undescribed dominant mouse model of cerebellar ...

In order to reveal non-neuronal cell interactions after peripheral nerve lesions, we began to analyze the impact of sciatic nerve fibroblasts on Schwann cells in vitro. Both cell types are considered to have opposite effects on axonal regeneration. Few data are available on how repulsive nerve fibroblasts affect neuritotrophic Schwann ...

Physiological cell death is crucial for matching defined cellular populations within the central nervous system. Whereas the time course of developmental cell death in the central nervous system is well analyzed, information about its precise spatial patterning is scarce. Yet, the latter one is needed to appraise its contribution to ...

STUDY DESIGN: Magnetically isolated, peripheral blood-derived CD133+ cells were used as the therapeutic agent of spinal cord injury (SCI). A rat model was used to investigate the hypothesis that the cell therapy using this clinically accessible cell fraction could be an attractive option for injured spinal cord. OBJECTIVE: Given the ...

Research on arterial chemoreceptors, particularly on the carotid body, has been fruitful in the last fifty years, to which this review is addressed. The functional anatomy of the organ appears to be well established. The biophysical bases by which glomus cells transduce chemical changes in the milieu intérieur (hypoxia, hypercapnia, ...

BACKGROUND: Immunoglobulin G (IgG) antibodies reactive with intracellular neuronal proteins have been described in paraneoplastic and other autoimmune disorders. Because neurons have been thought impermeable to immunoglobulins, however, such antibodies have been considered unable to enter neurons and bind to their specific antigens during life. Cerebellar Purkinje cells - an ...

Recent studies have demonstrated that the membrane potential of Purkinje cells is bi-stable and that this phenomenon underlies bi-modal simple spike firing. Membrane potential alternates between a depolarized state, that is associated with spontaneous simple spike firing (up state), and a quiescent hyperpolarized state (down state). A controversy has emerged ...

Calcium signaling plays a central role in normal CNS functioning and dysfunction. As cerebellar Purkinje cells express the major regulatory elements of calcium control and represent the sole integrative output of the cerebellar cortex, changes in neural activity- and calcium-mediated membrane properties of these cells are expected to provide important ...

The weeble mutant mouse has a frame shift mutation in inositol polyphosphate 4-phosphatase type I (Inpp4a). The phenotype is characterized by an early onset cerebellar ataxia and neurodegeneration, especially apparent in the Purkinje cells. Purkinje cell loss is a common pathological finding in many human and mouse ataxic disorders. Here ...

Purkinje cells are a class of specialized neurons in the cerebellum, and are among the most metabolically active of all neurons, as they receive immense synaptic stimulation, and provide the only efferent output from the cerebellum. Degeneration of Purkinje cells is a common feature of inherited ataxias in humans and ...

Tottering mouse is an ataxic mutant that carries a mutation in a gene encoding for the apha1A subunit of P/Q-type Ca2+ channel (Cav2.1). This study revisited to examine whether a Purkinje cell loss occurred in the cerebellum of tottering mice. In tottering mice, Calbindin D-28k negative gaps were apparent in ...

Low-density lipoprotein receptor-related protein (LRP-1) is an endocytic receptor for diverse proteins, including matrix metalloproteinase-9 (MMP-9), and a cell-signaling receptor. In the peripheral nervous system (PNS), LRP-1 is robustly expressed by Schwann cells only after injury. Herein, we demonstrate that MMP-9 activates extracellular-signal-regulated kinase (ERK1/2) and Akt in Schwann cells ...

The myelinating and nonmyelinating Schwann cells in peripheral nerves are derived from the neural crest, which is a transient and multipotent embryonic structure that also generates the other main glial subtypes of the peripheral nervous system (PNS). Schwann cell development occurs through a series of transitional embryonic and postnatal phases, ...

Ptychodera flava is a hemichordate whose anterior structures regenerate reproducibly from posterior trunk pieces when amputated. We characterized the cellular processes of anterior regeneration with respect to programmed cell death and cell proliferation, after wound healing. We found scattered proliferating cells at day 2 of regeneration using a proliferating cell ...

Svaren John J Department of Comparative Biosciences, School of Veterinary Medicine and Waisman Center, University of Wisconsin-Madison, Madison, Wisconsin, - - 2008

During late fetal life, Schwann cells in the peripheral nerves singled out by the larger axons will transit through a promyelinating stage before exiting the cell cycle and initiating myelin formation. A network of extra- and intracellular signaling pathways, regulating a transcriptional program of cell differentiation, governs this progression of ...

PURPOSE: The neurotrophic factor fibroblast growth factor-2 (FGF-2, bFGF) and Ca++ binding protein S100beta are expressed by the Schwann cells of the peripheral nerves and by the satellite cells of the dorsal root ganglia (DRG). Recent studies have pointed out the importance of the molecules in the paracrine mechanisms related ...

CONCLUSIONS: Schwann cells transfected by GDNF genes + PLGA were superior to Schwann cells + PLGA and direct anastomesis. This is a new and effective strategy for repair of facial nerve defects. OBJECTIVE: To evaluate the effect of bioactive artificial nerve conduits in the repair of facial nerve defects in ...

Postsynaptic density-95 (PSD-95) is one of neuronal nitric oxide synthase (nNOS)-anchoring proteins and plays an important role in specifying the sites of reaction of nitric oxide (NO) in the nervous system. The present study aims to investigate the presence of PSD-95 in rat Schwann cells (SCs) and the association of ...